Tricuspid atresia with pulmonary valve absence is a rare malformation characterized by left ventricular outflow obstruction due to asymmetrical ventricular septal hypertrophy and associated with a poor prognosis. Coexisting coronary-right ventricular fistula is rarely described. We encountered a case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricle fistula that successfully underwent Fontan completion. Right ventricle dilatation due to coronary-right ventricle fistula was observed in addition to mass like ventricular septal hypertrophy protruding into the left ventricular outflow tract. Right ventricle reduction and prevention of progressive left ventricular outflow obstruction were achieved by closure of the coronary-right ventricle fistula closure and plication of the right ventricle with the bidirectional Glenn procedure.

伴有肺动脉瓣缺失的三尖瓣闭锁是一种罕见的畸形，其特征是由于不对称室间隔肥厚导致左心室流出道梗阻，预后不良。很少描述共存的冠状右心室瘘。我们遇到了一例三尖瓣闭锁伴肺动脉瓣缺如合并冠状右心室瘘的病例，该病例成功地进行了 Fontan 完成。除了突出到左心室流出道的室间隔肥大状肿块外，还观察到由于冠状-右心室瘘引起的右心室扩张。